tbuckley/cabot-search huggingface dataset:
H10 An Oscar for Lassar
Authors: Huda Al-Kutubi, Maryam Salimi, Pooja Patel, Kashini Andrew, Natalie King-Stokes, Stephe Orpin
Journal: British Journal of Dermatology (2023)

Abstract Oscar Lassar (1849–1907) was born in Hamburg to a wealthy merchant family and received a top-class education. After enrolling in the school of medicine at Heidelberg, he volunteered for military service at the outbreak of the Franco–Prussian war in 1870. He served on the front line, was promoted to officer in reserves and decorated with the Iron Cross for his bravery. He returned to complete his medical training and would eventually open a dermatology hospital in Berlin, which unified patient care, research and teaching. In only a few years this hospital would become world renowned. Catering for both inpatient and outpatient care, he was able to offer a wide range of treatments, including complex topical therapy, electrotherapy and surgical procedures. His pasta salicylica (Lassar’s paste) remains to this day a great topical treatment for atopic dermatitis. Lassar recognized the importance of an experienced nursing team to support these activities. Furthermore, he understood the importance of a well-equipped dermatology department and was quick to acquire innovative technology to expand therapeutic options, including X-ray equipment for the treatment of tumours and a Finsen carbon arc lamp for treating lupus vulgaris, thus augmenting his reputation as a Wunderarzt. Lassar made great contributions towards solidifying dermatology as a highly regarded speciality through the establishment of journals and speciality societies. He involved himself in public health measures by campaigning for the establishment of public baths, which would allow even the poorest in society to enjoy clean water facilities at a low cost, thereby reducing the spread of infectious diseases. As a man, he was described as a larger-than-life character with a personal style that meant newspapers gladly reported on his activities, making him an excellent public relations master. Lassar’s recreation was chiefly riding and driving. He was an excellent linguist, an admirable host and fond of children, for whom he wrote Geschichten und Gedichten fiir kleine Kinder (Stories and Poems for Little Children). He died in an automobile accident and his burial on Christmas Eve was marked nationally by the German newspapers, and internationally by his patients and colleagues. In Lassar, we have a great historical example of effective visionary leadership that enabled the expansion of dermatological practice and the promotion of patient care at the time.
PA24 Abnormal interferon signature in a patient with chilblain lupus
Authors: Jaimie Oldham, Edel O’Toole
Journal: British Journal of Dermatology (2023)

Abstract A 5-year-old girl of Sri Lankan ancestry with no history of parental relatedness presented with the clinical features of chilblain lupus. This was particularly florid over her dorsal fingers, toes and the soles of her feet with areas in keeping with panniculitis on her calves and an epidermal naevus on the right side of her trunk. There was no ulceration or necrosis. Her parent reported occasional fevers and palpable lymph nodes in her neck. She also has bilateral astigmatism and mild valvular and supravalvular pulmonary stenosis. Blood tests showed a persisting mild transaminitis, an antinuclear antibody of 1 in 160 and a negative double-stranded DNA. She had a skin biopsy that showed supporting features for chilblain lupus. A lymph node biopsy showed nonspecific changes. The clinical suspicion was of a mild form of Aicardi–Goutières syndrome (AGS), and a genetic panel was performed that included TREX1, RNASEH2B, TMEM173 and SAMHD1 (all negative). The common mechanism behind these conditions is a type I interferonopathy. We sent blood on a research basis for RNA extraction and testing of her interferon (IFN) gene signature (IGS), which came back as abnormal (11.895; cutoff score of 2.758); she had normal expression of neutrophil-associated genes. This IFN score is on par with patients who have confirmed AGS or STING-associated vasculopathy with onset in infancy. An IGS is derived from the expression of IFN-regulated genes. A raised IGS has not only been reported to be useful as a biomarker in many rheumatic diseases, but is also used to help identify type I interferonopathies where genetic testing has not identified any clear pathogenic mutations (Crow YJ, Stetson DB. The type I interferonopathies: 10 years on. Nat Rev Immunol 2022; 22:471–83). National Institute for Health and Care Excellence guidelines allow for treatment of interferonopathies with baricitinib in patients with two documented high IFN gene signatures, which is our current plan for this patient.
GD05 ‘Ghosts’: changing perceptions of people with albinism in Tanzania
Authors: Stephanie Adeyemi
Journal: British Journal of Dermatology (2023)

Abstract Albinism, derived from the Latin word albus meaning white, is a rare genetic condition characterized by an absence of melanin leading to an individual having pale skin, eyes and hair. It is believed that Tanzania has the highest rate of albinism in Africa with the autosomal recessive gene being carried by one in 19 Tanzanians and the condition impacting one in 1400 people (Cruz-Inigo AE, Ladizinski B, Sethi A. Albinism in Africa: stigma, slaughter and awareness campaigns. Dermatol Clin 2011; 29:79–87). We aim to analyse the perception of individuals with albinism in Tanzania and strive to consider the future of those with albinism and reflect on the work being done. A recurring theme throughout the literature is the fact that those living with albinism in Tanzania are often shrouded in mysticism, stigma and violence. Referred to as ‘ghosts’, they are often on the fringes of society, ostracised by their communities and labelled as having supernatural powers. It is this superstition that has led to the brutal attack and murder of members of the disenfranchised albino population in Tanzania with the belief that their body parts, which can be sold for as high as $75 000, bring luck, as well as possessing powers. It is only in recent times that attention has been brought to their plight and work has begun to unravel these deeply entrenched beliefs. The United Nations (UN)-sanctioned International Albinism Awareness Day is on 13 June and aims to shed a light on the work being done to support people living with albinism globally. In 2013, the UN Human Rights Council adopted legislation to prevent violence against people with albinism, as well as a 2015 Mandate of an Independent Expert on the enjoyment of human rights by persons with albinism. A lot of work is being done on the ground by charitable organizations to empower people living with albinism in Tanzania through education and provision of safe shelter and resources. The role of dermatologists in the support of people with albinism in Tanzania cannot be ignored. Owing to decreased melanin levels in those with albinism, there is an increased risk of skin cancer due to a lack of protection against ultraviolent radiation. Dermatologists can play a crucial role, as part of a multidisciplinary team, in providing skin cancer prevention education, as well as providing education to reduce stigma against those living with albinism (McBride SR, Leppard BJ. Attitudes and beliefs of an albino population towards sun avoidance: advice and services provided by an outreach albino clinic in Tanzania. Arch Dermatol 2002; 138:629–32).
H14 ‘Reflection for the day’
Authors: Amy Long, Alison Long
Journal: British Journal of Dermatology (2023)

Abstract Thomas B. Fitzpatrick had a tremendous influence on the specialty of dermatology, including seminal scientific contributions, a legendary ability to teach and train, and remarkable innovations in clinical dermatology. Born in Wisconsin in 1919, he received his medical degree from Harvard, followed by a PhD in pathology. He served 2 years at an Army Chemical Centre, where his interest in skin pigmentation led to the discovery of human tyrosinase. Further innovation in basic sciences followed, with the discovery of the melanosome and epidermal melanin unit. He added to his academic accolades by completing a fellowship in chemistry at Oxford, then returned to the USA to pursue clinical dermatology training at Mayo Clinic. At the age of 32 years, following substantial success in his early career, he was appointed Professor and Chair of Dermatology at the University of Oregon. By 39 years of age, he was Chair of the Harvard Medical School Dermatology Department, Harvard’s youngest Professor and Chair, while serving as Chief of Dermatology at Massachusetts General Hospital. He was a master at motivating students and sharing his passion for dermatology. He often said ‘Practicing dermatologists are like the woodwind section of the orchestra—small in number, when they play they must play well’. In 1971, he wrote the first multiauthor medical textbook in the field entitled Fitzpatrick’s Dermatology in General Medicine, now in its eighth edition. Dr Fitzpatrick contributed heavily to the clinical advances in dermatology in the latter half of the 20th century. Alongside dermatopathologist Wallace Clark, he established the very first pigmented lesion clinic in 1966. From that clinic, studies enabled Dr Clark to devise the Clark level staging system for melanoma. Fitzpatrick described early diagnostic signs in melanoma, and the concept of sunlight as a factor in its aetiology. In 1975, he devised the Fitzpatrick scale of skin phototypes, which described a person’s skin type in terms of response to ultraviolet (UV) radiation exposure. To huge success, he experimented with psoralen and UVA to treat psoriasis, coining the term photochemotherapy, or PUVA. Fitzpatrick was a highly productive medical scholar, while having many other notable qualities and interests. He was an avid lover of music, a skilled pianist and a Johannes Brahms enthusiast. The Boston Globe called him a ‘dermatologist with an infectious enthusiasm for his specialty and philosopher’s love of a good quote’. For 20 years, he co-edited the Globe’s popular ‘Reflection for the Day’, alongside his loving wife Beatrice. Thomas Fitzpatrick was admired by many until his passing in 2003, leaving us with so much to remember him by.
BI03 (P76) Management of aciclovir-resistant mucocutaneous herpes simplex virus infection: a systematic review
Authors: Herbert Castillo Valladares, Nora Rudd, Kieron Leslie
Journal: British Journal of Dermatology (2023)

Abstract Aciclovir resistance results in immunocompromised hosts due to long-term antiviral therapy and ongoing viral replication. Aciclovir resistance in herpes simplex virus (HSV) poses a major concern, as this can lead to chronic and recalcitrant infection that significantly impacts on quality of life. While different treatment options exist, there are currently no standardized clinical treatment guidelines for mucocutaneous aciclovir-resistant (ACV-R) HSV infection. This systematic review seeks to identify safe and effective treatments for mucocutaneous ACV-R HSV infection. For this systematic review, PubMed/MEDLINE and Embase were searched in September 2022 for all articles published between 1 January 1980 and 1 September 2022. Studies were included if they described the use of medications for the treatment of mucocutaneous ACV-R HSV infection. From the initial search that yielded 1066 studies, 86 met the inclusion criteria and were selected. Of the 86 studies, six were clinical trials and 80 were case reports/series. A total of 318 patients were included in our review. Foscarnet [intravenous (IV) and topical] was studied in five clinical trials and 25 case reports/series, leading to complete response in most patients, with adverse effects including renal toxicity and electrolyte abnormalities. Vidarabine (IV) was found to be inferior to foscarnet in a clinical trial and reported in two case reports. Common side-effects included nausea, anaemia and renal impairment. Trifluridine (topical) was studied in a clinical trial and a case report, showing complete response in some patients. Cidofovir (IV, topical, intralesional) was studied in 21 case reports/series, showing complete response in most patients, with renal impairment as the most common adverse effect. Imiquimod (topical) was studied in eight case reports/series, showing complete response and no side-effects. Pritelivir (oral) was reported in two case reports/series, showing complete response and no side-effects. Combination treatment was reported in 10 case reports/series with variable clinical response. Our review also identified reports of less widely used treatments. These include artesunate, zidovudine, varicella-zoster virus vaccine, leflunomide and surgical excision, which achieved variable response rates but have not been widely studied. While foscarnet and cidofovir have the most robust evidence, IV administration of these agents is associated with significant side-effects and may be limiting. Intralesional and topical formulations of cidofovir, trifluridine and imiquimod are alternative options appropriate for outpatient management. Pritelivir is a novel and promising treatment of mucocutaneous ACV-R HSV infection.
BI17 A novel multidisciplinary clinic for the management of cutaneous T-cell lymphoma
Authors: Harry Heath, Emma Davies, Rainy Marsh, Claire Bailey
Journal: British Journal of Dermatology (2023)

Abstract Mycosis fungoides cutaneous T-cell lymphoma (MF-CTCL) is a rare condition that can present diagnostic difficulty and challenging management as no standard pathway exists. Patients require haematology and dermatology involvement to ensure optimal management, and the British Association of Dermatologists guidelines suggest regional supranetwork skin MDT for the majority of patients. However, this distances patients and local clinicians from the decision-making process. We propose a multidisciplinary model that includes local dermatology and haematology teams with the assistance of a regional expert to evaluate all of a region’s patients with MF-CTCL to ensure appropriate staging and management. On review of our cohort of patients with MF-CTCL, there were 42 patients with only 50% staged. We proposed a day-long multidisciplinary clinic model that enabled all patients with MF-CTCL to attend the dermatology department to receive a teaching session by trained nurses, a 30-minute consultation with a dermatology consultant with direct access to a specialist haematology consultant and a regional cutaneous lymphoma specialist, and access to on-the-day blood tests and diagnostic biopsies if needed. Twenty-six patients attended the clinic; 22 were confirmed to have MF-CTCL, 21 of whom were appropriately staged on the day. Ninety-five per cent of patients said they felt ‘extremely involved and informed’ about their management and treatment decisions after the clinic (vs. 19% preclinic). Ninety-five per cent would recommend the clinic to a family or friend, and 90% would attend a similar clinic in the future. From a clinician perspective, the survey responses showed high value in having repeated exposure to patients with CTCL, understanding the management options for MF-CTCL, face-to-face discussions with the multidisciplinary team (MDT) and longer clinic appointments. In conclusion, we offer this MDT clinic model as an efficient means for delivering patient-centred management and care, continuing education to local referring clinicians and ensuring close working relationships between dermatology and haematology. We propose running this clinic annually to ensure that all patients with MF-CTCL have appropriate investigation, staging and management pathways. The success of the clinic has led the department to adopt this model in other areas such as inflammatory conditions.
DP15 ‘Unwell water’: the potential importance of environmental factors in skin cancer in people of colour
Authors: Muhammad Hyder Junejo, Jeva Cernova, Marcin Ligaj, Matthew Stodell, Catherine Harwood
Journal: British Journal of Dermatology (2023)

Abstract Ultraviolet radiation (UVR) is the most common environmental carcinogen to cause cutaneous squamous cell carcinoma (cSCC). However, other carcinogens may also play a role and should be suspected when cSCC occurs in people with skin of colour (SOC) in whom ultraviolet (UV)-induced cSCC is less common. We present the case of a South Asian female who presented with an aggressive cSCC, which we hypothesize was related to previous arsenic exposure. A 70-year-old Bangladeshi woman (skin phototype V) presented with a 4-cm diameter, tender, keratotic plaque at the base of her left thumb, which had been present for 12 years and was slowly enlarging. There was no associated lymphadenopathy. She also had multiple palmoplantar keratotic papules and two hyperkeratotic plaques in UV-protected areas on her trunk. Histology of the lesion on her thumb confirmed a cSCC with high-risk features: poorly differentiated, invasion 9.5 mm into fibromuscular tissue, perineural invasion (nerve diameter &amp;gt; 0.1 mm) and vascular invasion. The truncal plaques were confirmed to be SCC in situ. The cSCC was incompletely excised and subsequently showed extensive involvement of periosteal soft tissues with invasion into bone marrow. Staging computed tomography showed no evidence of nodal or distant metastatic disease. She subsequently underwent ray amputation and remains under close clinical surveillance. Given the cSCC location and her skin phototype, potential non-UV radiation (UVR) causes were explored. In view of the palmoplantar keratotic papules and SCC in situ, chronic arsenic exposure was suspected. Further questioning revealed a history of drinking well water in a rural village in Bangladesh throughout childhood and early adulthood, although exposure had stopped &amp;gt; 40 years ago. Arsenic exposure from contaminated groundwater was therefore considered to be the most likely diagnosis. She was started on long-term chemoprevention with acitretin. Arsenic is a potent human carcinogen and a well-recognized cause of cSCC; the latency period may be 30–40 years. Most cases are likely to be in people exposed through environmental arsenic exposure, such as contaminated groundwater, with some cases of exposure to arsenic-containing traditional medicines also reported. Carcinogenic mechanisms are uncertain but may involve dysregulation of the Hippo signalling pathway. In summary, although the incidence of UVR-related cSCC is lower, this case highlights the importance of considering risk factors, other than UVR, in cSCC arising in people with SOC. Identifying relevant carcinogens may require a detailed travel, social and cultural history and, more importantly, an awareness of the non-UVR causes of cSCC.
BI27 <i>Strongyloides stercoralis</i> screening in dermatology
Authors: Jaimie Oldham, Jonathan Lambourne, Edel O’Toole
Journal: British Journal of Dermatology (2023)

Abstract Strongyloidiasis is a major neglected tropical disease and chronic parasitic infection caused by Strongyloides stercoralis. An estimated 30–100 million people are infected worldwide, with acquisition via contact with contaminated soil. Accurate data for the UK are unavailable, but there is a prevalence of &amp;gt; 20% in certain immigrant populations. Many cases are asymptomatic; without therapy, the infection may be lifelong. Testing is more common in dermatology since the advent of dupilumab as infection can be life-threatening in the immunocompromised. Symptoms reported with strongyloidiasis include not only pruritus and urticaria but also abdominal and respiratory issues. Standard diagnostic testing of strongyloidiasis relies on demonstration of the parasite in faeces, body fluids or tissue samples, although serology is most often used in dermatology. The aim of our study was to assess why dermatologists are ordering Strongyloides serology and whether the results could identify features of infection. All Strongyloides serology tests sent by dermatology from two centres from January 2021 to December 2022 were analysed. Data collected included demographics, reason for testing, symptoms, foreign travel, test results and treatment. In total, 187 tests were sent. Patient demographics are provided in Table 1. The reasons for testing were before starting dupilumab in 57.2% (n = 107), pruritus in 13.4% (n = 25) and urticaria in 3.2% (n = 6/187). There was a recorded history of other symptoms of strongyloidiasis in five patients, and a recorded history of foreign travel in four. Twelve per cent of results were positive (n = 23/187), 6.9% (n = 13) were borderline and 80.7% (n = 151) were negative. There was no major skew toward a reason for testing in those who were positive/borderline. Eight per cent (n = 3/36) of those with positive/borderline results had other recorded symptoms. Of the positive/borderline results, 31% (n = 11/36) were not treated (mostly because they did not go on to have immunosuppression), 28% (n = 10) were treated with a single dose of ivermectin, 31% (n = 11) with two doses and 8% (n = 3) with four doses. The main reason for Strongyloides testing in dermatology is before starting dupilumab. A low proportion of the patients with positive/borderline results had symptoms, although often this was not recorded. A higher proportion of individuals of Asian or African ancestry had positive/borderline results. We shall also discuss evidence behind the methods of and reasons for Strongyloides testing.Table 1Patient demographicsDemographicAll tests (n = 187)Positive/borderline tests (n = 36)Male115 (61.5)22 (61)Female72 (38.5)14 (39)White52 (27.8)5 (14)Asian95 (50.8)22 (61)Black21 (11.2)5 (14)Other/not stated19 (10.2)5 (14)Data are presented as n (%).